Sexual Behavioral Disinhibition Associated with Nucleus Lentiformis Lesion: A Forensic Neuroscience Perspective Through a Case.

Organic brain disturbances particularly related to frontal cortex structures and subcortical areas including the basal ganglia may play a role in behavioral disinhibition disorders. Kluver-Bucy syndrome (KBS), which is one of the better knowns of these syndromes, includes hyperorality, visual agnosia, and hypersexuality, has been reported to occur after temporal lobe and amygdala lesions; however, several patients who had focal lesions in areas other than the temporal cortex and amygdala have been reported to present partial KBS symptoms. Nucleus lentiformis refers to a large portion of the basal ganglia including the putamen and globus pallidus, and specific structures within this broad area are known to be important for reward and value-based decision making. To date, KBS symptoms including hypersexual behavior associated with nucleus lentiformis lesions have never been reported. Here, we present a 38-year-old male patient who developed increased sexual interest and hyperorality after infarctions in the right lenticular nucleus and right occipitotemporal region and committed a first-degree sexual assault. He was sent to our institution for the assessment of criminal responsibility to the index sexual crime. According to a comprehensive and thorough forensic psychiatric evaluation, he was diagnosed as having an organic personality disorder with partial KBS symptoms. To the best of our knowledge, this is the first reported case of deviant sexual behavior and hyperorality developing after nucleus lentiformis infarction. We aimed to discuss possible neurobiologic explanations of late-onset deviant sexual behavior, which resulted in sexual criminal behavior following a cerebrovascular infarction.

Clinical manifestations and morphological changes in one case with post-stroke Klüver-Bucy syndrome.

We present the case of a 71-year-old right-handed male, admitted to the Department of Neurology, Emergency County Hospital of Arad, Romania, on November 2015, with a rare case of Klüver-Bucy syndrome (KBS), following an ischemic stroke of the right temporal lobe, which was previously diagnosed in December 2014 and was treated accordingly. At the moment of second hospital admission, the patient was found somnolent and confused at home, with traumatic signs of biting of the tongue and urine emission. A couple days after admission, our patient became alert and presented hypersexuality, hypermetamorphosis, increased oral tendency, behavior changes including apathy with loss of anger and fear, and a very increased appetite, transient visual agnosia and right-left disorientation. In the initial phase, the patient could not recognize any members of his family, but he had a tendency to touch everything within his reach and place it into his mouth. The KBS presented in this case, following an ischemic stroke of the right temporal lobe provides distinct and intriguing insights into the possible pathophysiology of this syndrome. Often disruption of consciousness during recovery period may hide the clinical manifestation of the syndrome.

Klüver-Bucy syndrome associated with a recessive variant in HGSNAT in two siblings with Mucopolysaccharidosis type IIIC (Sanfilippo C).

Klüver-Bucy syndrome (KBS) comprises a set of neurobehavioral symptoms with psychic blindness, hypersexuality, disinhibition, hyperorality, and hypermetamorphosis that were originally observed after bilateral lobectomy in Rhesus monkeys. We investigated two siblings with KBS from a consanguineous family by whole-exome sequencing and autozygosity mapping. We detected a homozygous variant in the heparan-α-glucosaminidase-N-acetyltransferase gene (HGSNAT; c.518G>A, p.(G173D), NCBI ClinVar RCV000239404.1), which segregated with the phenotype. Disease-causing variants in this gene are known to be associated with autosomal recessive Mucopolysaccharidosis type IIIC (MPSIIIC, Sanfilippo C). This lysosomal storage disease is due to deficiency of the acetyl-CoA:α-glucosaminidase-N-acetyltransferase, which was shown to be reduced in patient fibroblasts. Our report extends the phenotype associated with MPSIIIC. Besides MPSIIIA and MPSIIIB, due to variants in SGSH and NAGLU, this is the third subtype of Sanfilippo disease to be associated with KBS. MPSIII should be included in the differential diagnosis of young patients with KBS.

[Klüver-Bucy syndrome after herpes simplex encephalitis]. / Klüver-Bucy-Syndrom nach Herpes-simplex-Enzephalitis.

We present the case of an 18-year-old women with Klüver-Bucy syndrome as sequela of Herpes simplex encephalitis. The symptomatology remitted partly under treatment with carbamazepine.

Mucopolysaccharidosis Type IIIA presents as a variant of Klüver-Bucy syndrome.

Mucopolysaccharidosis Type IIIA (MPS IIIA) is a neurodegenerative disease with behavioral symptoms unique among the mucopolysaccharidoses. Children with MPS IIIA reportedly mouth things, explore novel environments almost continuously, disregard danger, and empathize/socialize and comply less with parents. These characteristics resemble Klüver-Bucy syndrome (K-Bs). To test the K-Bs hypothesis, 30 children with MPS IIIA were compared to 8 "posttransplant" mucopolysaccharidosis Type IH patients in an experimental "risk room." The room contained attractive and mildly frightening objects, exposure to a 92-dB startle noise triggered by contact with an attractive toy, mother's return after a brief absence, and compliance with her cleanup directive. Children with MPS IIIA: (a) left mother sooner, (b) wandered more, (c) were more likely to approach frightening objects, (d) were less likely to respond to loud noise with whole body startle, (e) were less likely to avoid the toy associated with the startle noise, (f) interacted less with mother upon her return, and (g) complied less with her cleanup command. K-Bs is associated with loss of amygdala function. Brain magnetic resonance imaging (MRI) of a subset of the children with MPS IIIA showed volume loss that was greater in the amygdala than in the hippocampus; only amygdala loss correlated with reduced fearfulness. MPS IIIA may be the first identified pediatric disease presenting systematically as a K-Bs variant. If validated by further studies, the K-Bs hypothesis of MPS IIIA would provide important clinical and theoretical information for the guidance of families as well as markers for natural disease progression and treatment effects.

Relapsing herpes simplex encephalitis resulting in Kluver-Bucy syndrome.

Relapsing herpes simplex encephalitis (HSE) rarely occurs after acyclovir treatment. We treated a patient with relapsing HSE of the contralateral temporal lobe, resulting in Klüver-Bucy syndrome, after a full-dose acyclovir treatment. This case suggests that physicians should consider sudden behavioral and emotional changes after HSE treatment as a possible indication of relapsing HSE, as well as possible temporal lobe epilepsy, and the need to administer longer acyclovir treatment for select patients.

Klüver Bucy syndrome following hypoglycaemic coma in a patient with glycogen storage disease type Ib.

Patients with type I glycogen storage disease (GSD) have poor tolerance to fasting, sometimes less than 3 hours during infancy. Even though most patients are able, as they get older, to tolerate a longer fasting period, they are at permanent risk for fast-induced hypoglycaemia, even in adulthood. Klüver Bucy syndrome, is characterized by psychic blindness (inability to recognize familiar objects), hypermetamorphosis (strong tendency to react to visual stimulus), increased oral exploration, placidity, indiscriminate hyper-sexuality and change in dietary habits. In this case report, we describe the development of Klüver Bucy syndrome in a 28-year-old man with type Ib GSD, following prolonged and severe hypoglycaemia triggered by a common respiratory infection.

Partial Klüver-Bucy syndrome in a patient with acute disseminated encephalomyelitis.

The symptoms of Klüver-Bucy syndrome (KBS) include hyperorality, hypersexuality, visual agnosia, hypermetamorphosis and decreased motor or vocal reaction to fear- or anger-provoking stimuli. This syndrome has been associated with a wide variety of neurodegenerative disorders, as well as traumatic, non-traumatic and infectious brain injuries. We report an 11-year-old boy who developed a fairly classical presentation of KBS, presumably in the setting of post-infectious acute disseminated encephalomyelitis (ADEM). This patient's presentation is a reminder of this rare syndrome and extends the clinical manifestations of ADEM, which is a relatively more common condition.

Transient News Events Test: feasibility in assessment of post-temporal lobectomy remote memory deficits.

Although anterograde memory deficits are well documented in patients with epilepsy, the extent to which remote memory deficits occur is less clear. This is due in part to a lack of reliable methods for assessment. The present study examined the feasibility of using the Transient News Events Test (TNET) to assess remote memory in subjects status post anterior temporal lobectomy (ATL) for the treatment of refractory seizures. Results indicated significantly poorer performance of the patient group compared to healthy controls. The decrement in performance within the patient group was evident only for items from more recent time periods. Reasons for an apparent stability of the most remote memories with ATL and implications regarding hippocampal function are reviewed. In conclusion, the TNET provides a feasible method for assessment of remote memory function in patients with epilepsy, with decrements in performance noted in comparison to a healthy control group in this retrospective study.

[Klüver-Bucy syndrome. Seven year follow-up of one patient]. / Síndrome de Klüver-Bucy. Seguimiento de un paciente durante 7 años.

OBJECTIVE: To present a left-handed patient who had an acute encephalopathy, possibly of viral etiology, followed by remote and recent memory loss, several types of apraxia and emotional disturbance, without any motor abnormalities. He had cerebral lesions that involved both temporal lobes and other brain regions. All features corresponded to the Klüver-Bucy syndrome. After a seven year follow-up, no improvement of the neurological and neuroradiological, mainly by magnetic resonance imaging (MRI) features was observed. CASE REPORT: A 14 year-old left-handed boy suffered sudden onset of fever (40.4 degrees C), headache, vomiting, focal and generalized seizures and coma. After the acute illness, the patient had severe neurological sequels consisting in total loss of memory without any capacity to recognize persons (including family members) and remote events and he was not capability of remember hardly anything that he was taught after his disease, these alterations continued almost completely during the seven years (from 14 to 21 years) that we followed him up. The only abilities that he conserved in similar conditions to those prior to his acute disease were his capacity to swim (including the style of jumping into the water), bike riding, playing football, dominoes, cards, etc., which he had learned during his childhood, to say the numbers and the alphabet letters rapidly by heart (without knowing them) and to avoid cars on the street. MRI showed post-inflammatory lesions in the temporal and the parieto-temporo-occipital regions bilaterally (cortical and subcortical regions) and in the left occipital region. He presented almost all types of apraxia. CONCLUSION: Klüver-Bucy syndrome, which can be secondary to more than 50 different causes, not only presents remote memory loss but also recent memory loss as in our patient, who appeared to be isolated from the surrounding world. Motor function and automated activities learned before the acute brain illness were not affected and could be recovered. Etiology, location and extent of the anatomic brain lesion appear to be the most important prognostic conditions.

Síndrome de Klüver-Bucy. Seguimiento de un paciente durante 7 años / Klüver-Bucy syndrome. Seven year follow-up of one patient

Objetivo. Presentar a un paciente zurdo que sufrió un proceso agudo cerebral, posiblemente una encefalitis vírica, que le dejó alteraciones de la memoria para los hechos remotos y recientes, diferentes tipos de apraxia y falta de reacción afectiva, sin alteraciones motrices. Existía lesión cerebral en ambos lóbulos temporales y en otras zonas, todo ello compatible con síndrome de Klüver-Bucy. Su seguimiento durante 7 años apenas mostró mejoría de las alteraciones neurológicas y neurorradiológicas, principalmente resonancia magnética (RM). Caso clínico. Hombre que a los 14 años de edad presentó un cuadro brusco de fiebre alta (40,4 oC), cefalea, vómitos, convulsiones y coma. Tras pasar el proceso agudo, el paciente presentó secuelas neurológicas graves consistentes en absoluta falta de memoria sin capacidad alguna de reconocimiento de las personas (incluidos los miembros de la familia) y de hechos remotos, e incapacidad de recordar apenas nada de lo que se le iba enseñando tras su enfermedad, continuando con estas alteraciones, casi en su totalidad, a lo largo de los 7 años (desde los 14 hasta los 21 años) que seguimos su evolución. Sólo conservó, en condiciones similares a como lo realizaba antes de su enfermedad aguda, la capacidad para nadar (incluida la forma perfecta de tirarse al agua), para correr en bicicleta, jugar al fútbol, al dominó, a las cartas, etc., que él aprendió durante su infancia, decir los números y las letras del abecedario «de carrerilla» (sin reconocerlos cuando se le ponían delante) y para sortear los coches en la calle. La RM cerebral mostraba lesiones postinflamatorias en ambos lóbulos temporales, en ambas regiones parietotemporooccipitales (zonas corticales y subcorticales) y en la región occipital izquierda. Presentaba casi todos los tipos de apraxia. Conclusión. El síndrome de Klüver-Bucy, que puede ser secundario a más de 50 causas, no se limita a la pérdida de la memoria para los hechos remotos, sino que puede presentar también incapacidad para rememorar los conocimientos recientes, quedando los pacientes aislados mentalmente del mundo exterior. Su motricidad y la actividad automatizada, aprendida en épocas anteriores al proceso agudo que causó su encefalopatía, no fueron afectados y pudieron recuperarse. La etiología junto con la localización y extensión de las lesiones cerebrales parecen los factores pronósticos más importantes (AU)

Objective: To present a left-handed patient who had an acute encephalopathy, possibly of viral etiology, followed by remote and recent memory loss, several types of apraxia and emotional disturbance, without any motor abnormalities. He had cerebral lesions that involved both temporal lobes and other brain regions. All features corresponded to the Klüver-Bucy syndrome. After a seven year follow-up, no improvement of the neurological and neuroradiological, mainly by magnetic resonance imaging (MRI) features was observed. Case Report: A 14 year-old left-handed boy suffered sudden onset of fever (40.4 degrees C), headache, vomiting, focal and generalized seizures and coma. After the acute illness, the patient had severe neurological sequels consisting in total loss of memory without any capacity to recognize persons (including family members) and remote events and he was not capability of remember hardly anything that he was taught after his disease, these alterations continued almost completely during the seven years (from 14 to 21 years) that we followed him up. The only abilities that he conserved in similar conditions to those prior to his acute disease were his capacity to swim (including the style of jumping into the water), bike riding, playing football, dominoes, cards, etc., which he had learned during his childhood, to say the numbers and the alphabet letters rapidly by heart (without knowing them) and to avoid cars on the street. MRI showed post-inflammatory lesions in the temporal and the parieto-temporo-occipital regions bilaterally (cortical and subcortical regions) and in the left occipital region. He presented almost all types of apraxia. Conclusion: Klüver-Bucy syndrome, which can be secondary to more than 50 different causes, not only presents remote memory loss but also recent memory loss as in our patient, who appeared to be isolated from the surrounding world. Motor function and automated activities learned before the acute brain illness were not affected and could be recovered. Etiology, location and extent of the anatomic brain lesion appear to be the most important prognostic conditions (AU)

[Klüver-Bucy-like syndrome and frontal symptoms following cerebrovascular disease]. / Beyin Damar Hastaligi Sonrasi Gelisen Frontal Belirtiler ve Klüver-Bucy Benzeri Sendrom.

We present a case with frontal lobe symptoms and Klüver-Bucy-like syndrome following subarachnoid hemorrhage and hydrocephaly. Klüver-Bucy syndrome is a rare neurobehavioral condition characterized by placidity, visual agnosia, hypersexuality, hyperorality, and hypermetamorphosis (the tendency to react to or to touch every visual stimulus). The syndrome is usually associated with lesions of the amygdala or its pathways, and it occurs after head trauma, anoxia-ischemic encephalopathy, herpes simplex encephalitis, and Reye 's syndrome. A 45-year-old right-handed female patient, who developed hydrocephaly after meningitis due to bilateral middle cerebral artery aneurysm surgery presented to our psychiatry clinic with various behavioral and emotional changes. In her psychiatric examination, increased and disinhibited speech, perseveration, placidity, impaired go/no go task performance, and hyperphagia were observed. The patient was treated with risperidone 0.5 mg/day. Magnetic resonance imaging (MRI) of the brain showed encephalomalacic-gliotic changes in the anterior superior medial temporal lobe (including bilateral amygdala), hydrocephalus, bilateral abnormal signal intensity in the white matter of the frontal region, and bilateral infarction in the centrum semiovale. Symptoms, such as placidity (loss of anger and fear) and altered dietary habits are some of the clinical features of Klüver-Bucy syndrome, whereas disinhibition and perseveration are associated with prefrontal cortex dysfunction.

[Klüver-Bucy syndrome in humans]. / Klüver-Bucy-Syndrom beim Menschen.

The Klüver-Bucy syndrome (KBS) was first described in 1937 as an experimental neurobehavorial syndrome in monkeys with bitemporal brain lesions. The syndrome in man was subsequently observed to be transient or permanent in a variety of neurodegenerative disorders and after traumatic, nontraumatic, and infectious brain injury. Its most common manifestations are hyperorality with changes in dietary habits, hypersexuality, and visual agnosia. Seizures are another frequent symptom. Here we describe KBS in a female inpatient aged 30 in whom KBS and psychotic symptoms occurred together.

Pleomorphism of the clinical manifestations of neurocysticercosis.

Neurocysticercosis (NCC) remains a major public health problem in developing countries as it is the most common helminthic infection of the central nervous system. Clinical manifestations are non-specific and pleomorphic. Case reports on uncommon presentations of NCC are few. We report six interesting cases of NCC with unusual clinical presentation that demonstrate this spectrum of pleomorphism. These include extrapyramidal disease (parkinsonism and focal dystonia), Kluver-Bucy syndrome, Weber's syndrome, dementia and cortical blindness. The clinical details and possible mechanisms for the uncommon presentations are also discussed. Thus, a high level of suspicion should be kept for NCC, especially in endemic zones and developing countries.

Kluver-Bucy syndrome after mycoplasmal bronchitis.

Kluver-Bucy syndrome is a rare neurobehavioral condition characterized by visual agnosia, excessive oral tendencies, hypermetamorphosis, placidity, altered sexual behavior, and changes in dietary habits. This description of a 14-year-old boy presenting with complete Kluver-Bucy syndrome after Mycoplasma pneumoniae bronchitis is the first such case report. MRI revealed left temporal horn dilation and asymmetry of both temporal lobes. We hypothesize that the pathophysiology of our case is immune-mediated damage by M. pneumoniae resulting in vasculopathy.